scholarly journals Retrospective Analysis of INRG Clinical and Genomic Factors for 605 Neuroblastomas in Japan: A Report from the Japan Children’s Cancer Group Neuroblastoma Committee (JCCG-JNBSG)

Biomolecules ◽  
2021 ◽  
Vol 12 (1) ◽  
pp. 18
Author(s):  
Miki Ohira ◽  
Yohko Nakamura ◽  
Tetsuya Takimoto ◽  
Atsuko Nakazawa ◽  
Tomoro Hishiki ◽  
...  
Keyword(s):  
Survival Rate ◽  
Retrospective Analysis ◽  
Chromosome Aberrations ◽  
Primary Tumor ◽  
Survival Rates ◽  
Tumor Stage ◽  
Tumor Site ◽  
Primary Tumor Site ◽  
Cancer Group ◽  
Gain Loss

Neuroblastomas (NBs) exhibit broad and divergent clinical behaviors and tumor risk classification at diagnosis is crucial for the selection of an appropriate therapeutic strategy for each patient. The present study aimed to validate the clinical relevance of International Neuroblastoma Risk Group (INRG) prognostic and genomic markers in a Japanese NB cohort using a retrospective analysis. Follow-up data based on 30 common INRG queries in 605 NB cases diagnosed in Japan between 1990 and 2014 were collected and the genome signature of each tumor sample was integrated. As previously indicated, age, tumor stage, MYCN, DNA ploidy, the adrenals as the primary tumor site, serum ferritin and lactate dehydrogenase (LDH) levels, segmental chromosome aberrations, and the number of chromosome breakpoints (BP) correlated with lower survival rates, while the thorax as the primary tumor site and numerical chromosome aberrations correlated with a favorable prognosis. In the patient group with stage 4, MYCN non-amplified tumors (n = 225), one of the challenging subsets for risk stratification, age ≥ 18 months, LDH ≥ 1400 U/L, and BP ≥ 7 correlated with lower overall and event-free survival rates (p < 0.05). The genome subgroup GG-P2s (partial chromosome gain/loss type with 1p/11q losses and 17q gain, n = 30) was strongly associated with a lower overall survival rate (5-year survival rate: 34%, p < 0.05). Therefore, the combination of the tumor genomic pattern (GG-P2s and BP ≥ 7) with age at diagnosis and LDH will be a promising predictor for MYCN-non-amplified high-risk NBs in patient subsets, in accordance with previous findings from the INRG project.

scholarly journals Clinical Profiles and Survival Outcomes of Patients With Well-Differentiated Neuroendocrine Tumors at a Health Network in New South Wales, Australia: Retrospective Study

JMIR Cancer ◽  
10.2196/12849 ◽  
2019 ◽  
Vol 5 (2) ◽  
pp. e12849
Author(s):  
Jocelyn Reeders ◽  
Vivek Ashoka Menon ◽  
Anita Mani ◽  
Mathew George
Keyword(s):  
Survival Rate ◽  
Neuroendocrine Tumors ◽  
Primary Tumor ◽  
Univariate Analysis ◽  
Survival Outcomes ◽  
Tumor Site ◽  
Primary Tumor Site ◽  
Clinical Profiles ◽  
Well Differentiated ◽  
Related Mortality

Background Neuroendocrine tumors (NETs) are a heterogeneous group of malignancies with varying and often indolent clinicobiological characteristics according to their primary location. NETs can affect any organ and hence present with nonspecific symptoms that can lead to a delay in diagnosis. The incidence of NETs is increasing in Australia; data regarding characteristics of NETs were collected from the cancer registry of Hunter New England, Australia. Objective This study aimed to explore the clinical profiles and treatment and survival outcomes of patients with well-differentiated NETs in an Australian population. Methods We reviewed the data of all adult patients who received the diagnosis of NET between 2008 and 2013. The clinicopathological, treatment, and follow-up data were extracted from the local Cancer Clinical Registry. We also recorded the level of remoteness for each patient by matching the patient’s residential postcode to the corresponding Australian Bureau of Statistics 2011 remoteness area category. Univariate analysis was used to find the factors associated with NET-related mortality. Survival analysis was computed. Results Data from 96 patients were included in the study (men: 37/96, 38.5%, and women: 59/96, 61.5%). The median age at diagnosis was approximately 63 years. A higher proportion of patients lived in remote/rural areas (50/96, 52.1%) compared with those living in city/metropolitan regions (46/96, 47.9%). The most common primary tumor site was the gastroenteropancreatic tract, followed by the lung. The factors significantly associated with NET-related mortality were age, primary tumor site, surgical resection status, tumor grade, and clinical stage of the patient. At 5 years, the overall survival rate was found to be 62%, and the disease-free survival rate was 56.5%. Conclusions Older age, advanced unresectable tumors, evidence of metastasis, and higher-grade tumors were associated with poorer outcomes. Lung tumors had a higher risk of NET-related mortality compared with other sites.

scholarly journals Clinical Profiles and Survival Outcomes of Patients With Well-Differentiated Neuroendocrine Tumors at a Health Network in New South Wales, Australia: Retrospective Study (Preprint)

2018 ◽  
Author(s):  
Jocelyn Reeders ◽  
Vivek Ashoka Menon ◽  
Anita Mani ◽  
Mathew George
Keyword(s):  
Survival Rate ◽  
Neuroendocrine Tumors ◽  
Primary Tumor ◽  
Univariate Analysis ◽  
Survival Outcomes ◽  
Tumor Site ◽  
Primary Tumor Site ◽  
Clinical Profiles ◽  
Well Differentiated ◽  
Related Mortality

BACKGROUND Neuroendocrine tumors (NETs) are a heterogeneous group of malignancies with varying and often indolent clinicobiological characteristics according to their primary location. NETs can affect any organ and hence present with nonspecific symptoms that can lead to a delay in diagnosis. The incidence of NETs is increasing in Australia; data regarding characteristics of NETs were collected from the cancer registry of Hunter New England, Australia. OBJECTIVE This study aimed to explore the clinical profiles and treatment and survival outcomes of patients with well-differentiated NETs in an Australian population. METHODS We reviewed the data of all adult patients who received the diagnosis of NET between 2008 and 2013. The clinicopathological, treatment, and follow-up data were extracted from the local Cancer Clinical Registry. We also recorded the level of remoteness for each patient by matching the patient’s residential postcode to the corresponding Australian Bureau of Statistics 2011 remoteness area category. Univariate analysis was used to find the factors associated with NET-related mortality. Survival analysis was computed. RESULTS Data from 96 patients were included in the study (men: 37/96, 38.5%, and women: 59/96, 61.5%). The median age at diagnosis was approximately 63 years. A higher proportion of patients lived in remote/rural areas (50/96, 52.1%) compared with those living in city/metropolitan regions (46/96, 47.9%). The most common primary tumor site was the gastroenteropancreatic tract, followed by the lung. The factors significantly associated with NET-related mortality were age, primary tumor site, surgical resection status, tumor grade, and clinical stage of the patient. At 5 years, the overall survival rate was found to be 62%, and the disease-free survival rate was 56.5%. CONCLUSIONS Older age, advanced unresectable tumors, evidence of metastasis, and higher-grade tumors were associated with poorer outcomes. Lung tumors had a higher risk of NET-related mortality compared with other sites.

Comparison of treatment regimens for pediatric lymphoblastic non-Hodgkin's lymphoma: a Childrens Cancer Group study.

1995 ◽  
Vol 13 (6) ◽  
pp. 1368-1376 ◽  
Author(s):  
D G Tubergen ◽  
M D Krailo ◽  
A T Meadows ◽  
J Rosenstock ◽  
M Kadin ◽  
...  
Keyword(s):  
Primary Tumor ◽  
Hodgkin’S Lymphoma ◽  
Myelogenous Leukemia ◽  
Hodgkin's Lymphoma ◽  
Tumor Site ◽  
Primary Tumor Site ◽  
Non Hodgkin’S Lymphoma ◽  
Non Hodgkin's Lymphoma ◽  
Localized Disease ◽  
Extent Of Disease

PURPOSE Patients with lymphoblastic non-Hodgkin's lymphoma (LB NHL) were randomized to treatment with either modified LSA2L2 or ADCOMP, which added daunorubicin (DAUN) and asparaginase (L-ASP) to the methotrexate (MTX), cyclophosphamide (CYT), vincristine (VCR), and prednisone (PRED) (COMP) regimen, in a clinical trial to determine the relative effectiveness and toxicity of the two regimens. PATIENTS AND METHODS Patients with LB NHL were eligible for this randomized study if they were less than 22 years of age at diagnosis and had < or = 25% blasts in the bone marrow. Of 307 patients registered, 281 were fully eligible and assessable. Patients were stratified by extent of disease at diagnosis. RESULTS The 5-year event-free survival (EFS) rate for patients with localized disease was 84%, and for patients with disseminated disease, 67%. There were four relapses in 28 patients with localized disease. Two hundred six patients had mediastinal primary tumors and despite local radiation, 34 of 63 failures in these patients involved the primary tumor site with or without other involvement. After adjusting for extent of disease at diagnosis, the regimens did not differ significantly with respect to risk for adverse events. The acute toxicity was primarily neutropenia and thrombocytopenia, with greater initial toxicity in patients on the LSA2L2 regimen. Three patients developed acute myelogenous leukemia. CONCLUSION Long-term EFS in children with LB NHL can be achieved in the majority of patients. Disease progression, which includes recurrence at the primary tumor site, is a major cause of treatment failure in patients with mediastinal presentations. Addition of DAUN and L-ASP to the COMP regimen does not produce a more effective treatment than LSA2L2.

A phase II/III randomized double-blind study of octreotide acetate LAR with axitinib versus octreotide acetate LAR with placebo in patients with advanced G1-G2 NETs of non-pancreatic origin (AXINET trial-GETNE-1107).

2021 ◽  
Vol 39 (3_suppl) ◽  
pp. 360-360
Author(s):  
Rocio Garcia-Carbonero ◽  
Marta Benavent ◽  
Paula Jiménez Fonseca ◽  
Daniel Castellano ◽  
Teresa Alonso ◽  
...  
Keyword(s):  
Disease Progression ◽  
Phase Ii ◽  
Primary Tumor ◽  
Phase Iii ◽  
Study Entry ◽  
Tumor Site ◽  
Double Blind ◽  
Primary Tumor Site ◽  
Octreotide Lar ◽  
Octreotide Acetate

360 Background: Angiogenesis plays an important role in NET development and progression. Axitinib is a potent and selective VEGFR-1,2,3 inhibitor, with proven activity against several vascular-dependent solid tumors. The aim of this randomized, double-blind phase II/III study was to assess the efficacy of axitinib in patients with advanced G1-2 extra-pancreatic NETs. Methods: Eligible pts were randomized (1:1) to receive octreotide LAR (30 mg IM q4w) with axitinib (5 mg BID) or placebo BID until disease progression or unacceptable toxicity. Pteswere stratified by time from diagnosis to study entry ( > or < 12m), primary tumor site (GI tract vs non-GI) and Ki-67 index (< 5% vs > 5%). Prior therapy with SSA, IFN and up to 2 lines of systemic treatment was allowed, but not prior VEGF- or VEGFR-targeted drugs. Clinical and/or radiological disease progression within 12 months prior to study entry was required. The primary endpoint was progression-free survival (PFS). Secondary endpoints included overall survival (OS), time to progression, overall response rate (ORR), duration of response, biochemical response and safety. Results: 256 pts were randomized (106 in the Phase II part, and 150 additional pts in the Phase III part), 126 to axitinib and 130 to placebo. The main characteristics of the study population were: median age 61 years (range: 21-85), 52% male, PS 0-1 (64-35%), G1-2 (29%-71%), primary tumor site GI (40%)-Lung (17%)-Other (32%). Prior therapies included: SSA (46%), everolimus (13%), chemotherapy (13%), TACE (5%) and PRRT (2%). ORR was significantly higher in axitinib- vs placebo-treated patients (17.5% vs 3.8%, p = 0.0004). PFS per investigator assessment also favored axitinib vs placebo-treated patients, although the difference did not reach statistical significance (median PFS 17.2 vs 12.3 months, respectively, HR 0.816, p = 0.169). Grade 3-4 treatment-related AEs occurred more frequently in the axitinib vs placebo arm (52% vs 13.8%), and included hypertension (21% vs 6 %), cardiac disorders (3.2% vs 0.7%), diarrhoea (13% vs 1.5 %), asthenia (9% vs 3%) and nausea&vomiting (2% vs 0.7%). There were 3 treatment-related deaths, 1 in the axitinib arm (cardiac failure) and 2 in the placebo arm (myocardial infarction and hepatorenal syndrome). Conclusions: Although the study failed to demonstrate a significant PFS benefit per investigator assessment, axitinib in combination with octreotide LAR demonstrated activity and had a tolerable safety profile in patients with advanced G1-2 extra-pancreatic NETs. Data base cleaning and central blinded radiological PFS assessment are currently ongoing. Clinical trial information: NCT01744249.

Mucoepidermoid carcinoma: Evaluating the prognostic impact of primary tumor site

Oral Oncology ◽  
2021 ◽  
Vol 123 ◽  
pp. 105602
Author(s):  
Ximena Mimica ◽  
Avery Yuan ◽  
Ashley Hay ◽  
Nora Katabi ◽  
Daniella Karassawa Zanoni ◽  
...  
Keyword(s):  
Primary Tumor ◽  
Mucoepidermoid Carcinoma ◽  
Prognostic Impact ◽  
Tumor Site ◽  
Primary Tumor Site

Treatment of Children With Medulloblastomas With Reduced-Dose Craniospinal Radiation Therapy and Adjuvant Chemotherapy: A Children's Cancer Group Study

1999 ◽  
Vol 17 (7) ◽  
pp. 2127-2127 ◽  
Author(s):  
Roger J. Packer ◽  
Joel Goldwein ◽  
H. Stacy Nicholson ◽  
L. Gilbert Vezina ◽  
Jeffrey C. Allen ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Adjuvant Chemotherapy ◽  
Progressive Disease ◽  
Survival Rates ◽  
Progression Free Survival ◽  
Tumor Site ◽  
Local Tumor ◽  
Reduced Dose ◽  
Craniospinal Radiation ◽  
Cancer Group

PURPOSE: Medulloblastoma is the most common malignant brain tumor of childhood. After treatment with surgery and radiation therapy, approximately 60% of children with medulloblastoma are alive and free of progressive disease 5 years after diagnosis, but many have significant neurocognitive sequelae. This study was undertaken to determine the feasibility and efficacy of treating children with nondisseminated medulloblastoma with reduced-dose craniospinal radiotherapy plus adjuvant chemotherapy. PATIENTS AND METHODS: Over a 3-year period, 65 children between 3 and 10 years of age with nondisseminated medulloblastoma were treated with postoperative, reduced-dose craniospinal radiation therapy (23.4 Gy) and 55.8 Gy of local radiation therapy. Adjuvant vincristine chemotherapy was administered during radiotherapy, and lomustine, vincristine, and cisplatin chemotherapy was administered during and after radiation. RESULTS: Progression-free survival was 86% ± 4% at 3 years and 79% ± 7% at 5 years. Sites of relapse for the14 patients who developed progressive disease included the local tumor site alone in two patients, local tumor site and disseminated disease in nine, and nonprimary sites in three. Brainstem involvement did not adversely affect outcome. Therapy was relatively well tolerated; however, the dose of cisplatin had to be modified in more than 50% of patients before the completion of treatment. One child died of pneumonitis and sepsis during treatment. CONCLUSION: These overall survival rates compare favorably to those obtained in studies using full-dose radiation therapy alone or radiation therapy plus chemotherapy. The results suggest that reduced-dose craniospinal radiation therapy and adjuvant chemotherapy during and after radiation is a feasible approach for children with nondisseminated medulloblastoma.

scholarly journals Modeling tissue contamination to improve molecular identification of the primary tumor site of metastases

2014 ◽  
Vol 30 (10) ◽  
pp. 1417-1423 ◽  
Author(s):  
Martin Vincent ◽  
Katharina Perell ◽  
Finn Cilius Nielsen ◽  
Gedske Daugaard ◽  
Niels Richard Hansen
Keyword(s):  
Molecular Identification ◽  
Primary Tumor ◽  
Tumor Site ◽  
Primary Tumor Site ◽  
Tissue Contamination

Two case reports of rare diseases occurring in rare parts: Splenic vein solitary fibrous tumor and Liver solitary fibrous tumor

2020 ◽  
Author(s):  
Wenjing Wang ◽  
Banghe Bao ◽  
Anbin Hu ◽  
Xiaofeng Zhu ◽  
Qing Chen
Keyword(s):  
Liver Transplantation ◽  
Surgical Resection ◽  
Orthotopic Liver Transplantation ◽  
Solitary Fibrous Tumor ◽  
Primary Tumor ◽  
Splenic Vein ◽  
Tumor Site ◽  
Primary Tumor Site ◽  
Fibrous Tumor

Abstract Background Solitary fibrous tumor (SFT) is a rare soft tissue tumor originating from mesenchyme. Two cases of SFT we report right now occurred in the splenic vein and liver respectively, this primary splenic vein SFT may be the first report case, and also the first report of liver recurrence SFT cured by orthotopic liver transplantation (OLT). Case presentation One case was a 37-year-old female patient whose primary tumor site was located in the splenic vein, which resulted in splenomegaly and hypersplenism; its recurrence again and again after surgical resection and eventually transferred to the liver, during 10 years of follow-up, 4 operations were performed, and he is in a good condition right now. The second case was a 54-year-old male patient whose primary tumor site was located in the liver, spleen and left side of the chest wall; however, he had no uncomfortable symptoms. Surgeons performed two operations to remove these tumors, totally. 6 years later, SFT recurrence in the liver, and given that the tumor was so large that it could not be completely surgical resected, we chose orthotopic liver transplantation (OLT), and no tumor recurrence during 12-month follow-up. Conclusion The reports of these two cases of SFT are very rare, especially the splenic vein SFT, which expand the understanding of SFT. The main treatment of SFT is still surgical resection, right now, and liver transplantation may be a new option treatment for the huge liver SFT.

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